Biogen Idec Research Ltd.

The overall objective of this study was to evaluate the efficacy and safety of BIIB059 compared with placebo in subjects with active SCLE and/or CCLE (with or without systemic manifestations) who had an inadequate response to and/or intolerance to antimalarial therapy.

This study is a multicenter, randomized, parallel-group, dose-blinded, Phase III LTE study that will be conducted in subjects who have completed 52 weeks of study treatment in Study 230LE303 or Study 230LE304 (referred to as the “Phase III Parent Study” in this study protocol).

1. To evaluate the long-term safety and tolerability of higher doses of intrathecal nusinersen in SMA subjects who had previously participated in Study 203 2. To evaluate the long-term efficacy of higher doses of intrathecal nusinersen in SMA subjects who had previously participated in Study 203 3. To investigate the PK (cerebrospinal fluid and plasma) of intrathecal nusinersen at higher doses in SMA subjects who had previously participated in Study 203 4. To investigate the effect of nusinersen treatment on pNF-H levels (cerebrospinal fluid and plasma) after intrathecal administration of higher doses of nusinersen to SMA subjects who had previously participated in Study 203